FITC标记的原纤维蛋白样蛋白DHRD抗体
产品名称： FITC标记的原纤维蛋白样蛋白DHRD抗体
英文名称： Anti-EFEMP1/FITC
产品编号： HZ-14516R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-EFEMP1/FITC Conjugated antibody
FITC标记的原纤维蛋白样蛋白DHRD抗体
| 英文名称 | Anti-EFEMP1/FITC |
| 中文名称 | FITC标记的原纤维蛋白样蛋白DHRD抗体 |
| 别 名 | DHRD; Doyne honeycomb retinal degeneration; DRAD; EFEMP 1; EFEMP1; EGF containing fibulin like extracellular matrix protein 1; EGF containing fibulin like extracellular matrix protein 1 precursor; EGF-containing fibulin-like extracellular matrix protein 1; Epidermal growth factor containing fibulin like extracellular matrix protein 1; Extracellular protein S1 5; Extracellular protein S1-5; FBLN 3; FBLN3; FBLN3_HUMAN; FBNL; FIBL 3; FIBL-3; FIBL3; Fibrillin like; Fibrillin like protein; Fibrillin-like protein; FIBULIN 3; Fibulin-3; Fibulin3; FLJ35535; Heat shock 70 KD protein 1; MGC111353; MLVT; MTLV; S1 5; T16 protein. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 神经生物学 信号转导 细胞外基质 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 53kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human EFEMP1 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: This gene encodes a member of the fibulin family of extracellular matrix glycoproteins. Like all members of this family, the encoded protein contains tandemly repeated epidermal growth factor-like repeats followed by a C-terminus fibulin-type domain. This gene is upregulated in malignant gliomas and may play a role in the aggressive nature of these tumors. Mutations in this gene are associated with Doyne honeycomb retinal dystrophy. Alternatively spliced transcript variants that encode the same protein have been described.[provided by RefSeq, Nov 2009] Function: Binds EGFR, the EGF receptor, inducing EGFR autophosphorylation and the activation of downstream signaling pathways. May play a role in cell adhesion and migration. May function as a negative regulator of chondrocyte differentiation. In the olfactory epithelium, it may regulate glial cell migration, differentiation and the ability of glial cells to support neuronal neurite outgrowth. Subcellular Location: Secreted > extracellular space. Secreted > extracellular space > extracellular matrix. Localizes to the lamina propria underneath the olfactory epithelium. Tissue Specificity: In the eye, associated with photoreceptor outer and inner segment regions, the nerve fiber layer, outer nuclear layer and inner and outer plexiform layers of the retina. DISEASE: Defects in EFEMP1 are a cause of Doyne honeycomb retinal dystrophy (DHRD) [MIM:126600]; also known as malattia leventinese (MLVT) (ML). DHRD is an autosomal dominant disease characterized by yellow-white deposits known as drusen that accumulate beneath the retinal pigment epithelium. Similarity: Belongs to the fibulin family. Contains 6 EGF-like domains. Database links: Entrez Gene: 2202 Human Entrez Gene: 216616 Mouse Entrez Gene: 305604 Rat Omim: 601548 Human SwissProt: Q12805 Human SwissProt: Q8BPB5 Mouse SwissProt: O35568 Rat Unigene: 76224 Human Unigene: 44176 Mouse Unigene: 163265 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因编码腓肠素家族的细胞外基质糖蛋白的成员。和这个家族的所有成员一样，编码蛋白包含连续重复的表皮生长因子样重复序列，接着是C端纤维蛋白型结构域。该基因在恶性胶质瘤中上调，并可能在这些肿瘤的侵袭性中发挥作用。该基因突变与多伊-蜂窝状视网膜营养不良有关。可替代地，编码相同蛋白质的剪接转录变体已被描述。[由RefSeq，NOV 2009提供]